HLH

HLH or Hemophagocytic lymphohistiocytosis is a rare but potentially fatal condition that affects the immune system. It is characterized by the overproduction of activated immune cells, known as macrophages and lymphocytes, which can cause damage to tissues and organs.

Symptoms

The symptoms of HLH can vary widely, but often include fever, enlarged spleen (splenomegaly), enlarged liver (hepatomegaly), and skin rash. Other symptoms may include anemia, low platelet count (thrombocytopenia), and low white blood cell count (neutropenia).

Causes

HLH can be caused by certain genetic mutations, which are usually inherited from parents. These mutations affect the normal functioning of the immune system, leading to the overproduction of immune cells. HLH can also be triggered by certain infections, cancers, or autoimmune diseases.

Diagnosis

Diagnosis of HLH is often challenging due to the wide range of symptoms and their similarity to other conditions. It typically involves a combination of clinical findings, laboratory tests, and sometimes, a bone marrow biopsy.

Treatment

Treatment for HLH aims to reduce the overactive immune response and manage the symptoms. This often involves medications to suppress the immune system, and in severe cases, a bone marrow transplant may be necessary.

See also

• Macrophage
• Lymphocyte
• Bone marrow transplant

References

HLH Resources
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